Dayan A D
Postgrad Med J. 1969 Jun;45(524):401-7. doi: 10.1136/pgmj.45.524.401.
Subacute sclerosing panencephalitis is a rare disorder of late childhood and early adolescence Affected patients usually show behavioural and intellectual disturbance and involuntary movements before dying in coma after about 12 months. At some stage most have characteristic electroencephalographic abnormalities. Pathologically, changes in the brain are those of subacute encephalitis with a variable gliosis of the white matter, and sometimes intranuclear inclusion bodies in neurones and glial cells. Recent studies in many patients have shown high levels of circulating anti-measles antibodies, measles antigen in cells in the brain, and sometimes, myxo-virus filaments in cells there. These findings suggest that SSPE may be a slow measles virus infection of the nervous system. Possible explanations for the slow evolution of the encephalitis include disordered immune mechanisms and intracellular persistence of virus in a defective phase.
亚急性硬化性全脑炎是一种发生于儿童晚期和青春期早期的罕见疾病。受影响的患者通常在约12个月后昏迷死亡前出现行为和智力障碍以及不自主运动。在某个阶段,大多数患者会出现特征性的脑电图异常。病理上,脑部变化为亚急性脑炎,伴有白质不同程度的胶质增生,有时神经元和胶质细胞内可见核内包涵体。最近对许多患者的研究表明,循环抗麻疹抗体水平升高、脑内细胞中有麻疹抗原,有时脑内细胞中还有黏液病毒细丝。这些发现提示亚急性硬化性全脑炎可能是一种神经系统的慢麻疹病毒感染。脑炎缓慢发展的可能解释包括免疫机制紊乱以及病毒在缺陷阶段的细胞内持续存在。